RUBCN

Chr 3

rubicon autophagy regulator

Also known as: KIAA0226, RUBICON, SCAR15

The protein encoded by this gene is a negative regulator of autophagy and endocytic trafficking and controls endosome maturation. This protein contains two conserved domains, an N-terminal RUN domain and a C-terminal DUF4206 domain. The RUN domain is involved in Ras-like GTPase signaling, and the DUF4206 domain contains a diacylglycerol (DAG) binding-like motif. Mutation in this gene results in deletion of the DAG binding-like motif and causes a recessive ataxia. Alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, Apr 2014]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSpinocerebellar ataxia, autosomal recessive, 15
1
Active trials
12
Pubs (1 yr)
P/LP submissions
P/LP missense
0.79
LOEUF
DN
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.79LOEUF
pLI 0.000
Z-score 2.78
OE 0.58 (0.430.79)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
1.47Z-score
OE missense 0.83 (0.770.89)
472 obs / 570.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.58 (0.430.79)
00.351.4
Missense OE?0.83 (0.770.89)
00.61.4
Synonymous OE?0.92
01.21.6
LoF obs/exp: 29 / 50.3Missense obs/exp: 472 / 570.7Syn Z: 0.98

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RUBCN · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.